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Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports

BACKGROUND: McCune-Albright syndrome (MAS) is a rare multisystem disorder that classically was defined by the triad of polyostotic fibrous dysplasia of bone, café-au-lait skin pigmentation, and precocious puberty. It is a condition that has a gradual onset, slow growth rate and remain painless throu...

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Autores principales: Kabali, Theodora Miti, Moshy, Jeremiah Robert, Owibingire, Sira Stanslaus, Sohal, Karpal Singh, Simon, Elison N. M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6686505/
https://www.ncbi.nlm.nih.gov/pubmed/31395055
http://dx.doi.org/10.1186/s12903-019-0872-8
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author Kabali, Theodora Miti
Moshy, Jeremiah Robert
Owibingire, Sira Stanslaus
Sohal, Karpal Singh
Simon, Elison N. M.
author_facet Kabali, Theodora Miti
Moshy, Jeremiah Robert
Owibingire, Sira Stanslaus
Sohal, Karpal Singh
Simon, Elison N. M.
author_sort Kabali, Theodora Miti
collection PubMed
description BACKGROUND: McCune-Albright syndrome (MAS) is a rare multisystem disorder that classically was defined by the triad of polyostotic fibrous dysplasia of bone, café-au-lait skin pigmentation, and precocious puberty. It is a condition that has a gradual onset, slow growth rate and remain painless throughout. The clinical phenotype of MAS is highly variable and no definite treatment is available. CASE PRESENTATION: This article describes two cases, a 10-year-old girl and an 11-year-old boy, both with MAS comprising deforming craniofacial FD. Challenges related to diagnosis and management included late reporting with big lesions, involvement of multiple craniofacial bones, mutilating surgeries and ultimately high degree of morbidity. CONCLUSION: Delayed diagnosis and management of MAS results in devastating physical disabilities and severe morbidity after treatment.
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spelling pubmed-66865052019-08-12 Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports Kabali, Theodora Miti Moshy, Jeremiah Robert Owibingire, Sira Stanslaus Sohal, Karpal Singh Simon, Elison N. M. BMC Oral Health Case Report BACKGROUND: McCune-Albright syndrome (MAS) is a rare multisystem disorder that classically was defined by the triad of polyostotic fibrous dysplasia of bone, café-au-lait skin pigmentation, and precocious puberty. It is a condition that has a gradual onset, slow growth rate and remain painless throughout. The clinical phenotype of MAS is highly variable and no definite treatment is available. CASE PRESENTATION: This article describes two cases, a 10-year-old girl and an 11-year-old boy, both with MAS comprising deforming craniofacial FD. Challenges related to diagnosis and management included late reporting with big lesions, involvement of multiple craniofacial bones, mutilating surgeries and ultimately high degree of morbidity. CONCLUSION: Delayed diagnosis and management of MAS results in devastating physical disabilities and severe morbidity after treatment. BioMed Central 2019-08-08 /pmc/articles/PMC6686505/ /pubmed/31395055 http://dx.doi.org/10.1186/s12903-019-0872-8 Text en © The Author(s). 2019 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Kabali, Theodora Miti
Moshy, Jeremiah Robert
Owibingire, Sira Stanslaus
Sohal, Karpal Singh
Simon, Elison N. M.
Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports
title Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports
title_full Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports
title_fullStr Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports
title_full_unstemmed Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports
title_short Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports
title_sort craniofacial fibrous dysplasia associated with mccune-albright syndrome: challenges in diagnosis and treatment: case reports
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6686505/
https://www.ncbi.nlm.nih.gov/pubmed/31395055
http://dx.doi.org/10.1186/s12903-019-0872-8
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